Oncocytic papillary cystadenoma of the larynx: a case report.

BACKGROUND: Cystadenoma of the salivary glands is a rare benign clinical condition affecting both major and minor salivary glands equally. It constitutes approximately 2% of total neoplasms and 4.2-4.7% of benign formations in minor salivary glands. Typically presenting as a slow-growing, painless neoplasm, it can be distinguished from Cystadenolymphoma (Whartin's Tumor) by the absence of lymphoid elements in histological examination. While mostly located in the oral cavity and oropharynx, it can also be found in sinonasal mucosa, and rare cases have been identified in the larynx. CASE PRESENTATION: A 75-year-old Caucasian woman presented to the ear, nose, and throat department with complaints of dysphonia and headaches persisting for several months. Dysphonia had developed months after an unspecified vocal cord surgery elsewhere. Flexible laryngoscopy identified a left-sided cystic swelling affecting the supraglottic space, leading to respiratory obstruction and dysphonia. Head and neck computed tomography confirmed a 1.9 × 1.7 cm bilobed cystic mass originating from the left Morgagni ventricle. Microlaryngoscopy with CO2 laser excision and biopsy revealed a histopathological diagnosis of oncocytic papillary cystadenoma. Post-surgery, the patient fully recovered from dysphonia, with no significant complications noted. Long-term clinical surveillance was advised to detect potential recurrences promptly. CONCLUSION: Ectopic minor salivary gland tumors, both benign and malignant, should be taken into consideration as potential differential diagnosis for any swelling arising within the upper digestive tract mucosa. Ears, nose, and throat clinical examination completed by videolaryngoscopy can easily point out the location of the mass. Imaging is mandatory for differential diagnosis and for surgical planning. Surgical excision can provide both diagnosis and definitive cure.

Papillary cystadenoma of the major salivary gland: A case report and review of literature.

Papillary cystadenoma (PC) of the salivary gland is an uncommon benign epithelial neoplasm that shows predominantly multicystic growth pattern with intraluminal papillary proliferation and areas of oncocytic differentiation. We report a case of papillary cystadenoma of the parotid gland in a 44-years-old female. The patient presented with painful nodular swelling in the right parotid region for two months. Ultrasonography revealed a well marginated oval lesion with altered signal intensity involving the superficial lobe. The excision specimen showed a neoplasm with multicystic spaces having papillary projections lined by benign low-grade epithelium and supported by fibrovascular cores. No significant cytological atypia or mitosis was observed. The cells were immunoreactive for Keratin, Keratin 7, and were negative for Keratin 20, AR, HeR2/neu, TTF1, CDX2, and GATA3. p63 and Keratin 5/6 highlighted the myoepithelial cell layer lining the cystic spaces as well as the papillary projections. The Ki-67 proliferation index was 6%. The patient is on close clinical and imaging follow-up for the last 1year and 8 months without any evidence of disease recurrence or metastasis. Rarity of the lesion and distinct histomorphology warrants appropriate knowledge and discussion of the subject.

Oncocytic papillary cystadenoma of the larynx: comparative study of ten cases and review of the literature.

OBJECTIVE: Oncocytic papillary cystadenomas (OPCs) of the larynx are rare benign cystic lesions that usually present as supraglottic masses arising from the laryngeal ventricles. OPCs are found in patients older than 60 years, with a female predominance. Symptoms vary from asymptomatic to hoarseness, dyspnea, and dysphagia; often, they mimic a laryngocele. The treatment is surgical. Diagnosis is based on histopathologic examination. MATERIALS AND METHODS: Surgical records for laryngeal masses diagnosed between 2005 and 2020 were searched retrospectively. RESULTS: Ten patients were identified and included in the study. OPCs predominantly occurred in women (9/10), and the mean age at presentation was 73 years. Most patients (8/10) presented with hoarseness and were smokers. OPCs were localized in the ventricle in eight out of ten patients. Surgical treatment was performed in all cases, mostly using transoral endolaryngeal approach (9/10). Histopathologic examination revealed oncocytic cyst or oncocytic papillary cystadenoma (the former term being the older synonym for OPC). CONCLUSION: OPCs present a separate clinicopathologic entity, distinct from other cystic laryngeal lesions. They have a characteristic location, age and sex group, microscopic appearance, and potential for local recurrence.

Clear Cell Papillary Cystadenoma of the Ovary Masquerading as Metastatic Clear Cell Renal Cell Carcinoma: A Case Report and Review of the Literature.

Clear cell papillary cystadenoma of the epididymis is an uncommon benign neoplasm, usually seen in patients with von Hippel-Lindau disease. Morphologic and immunohistochemical examination aid in distinguishing clear cell papillary cystadenoma from malignant histologic mimics including low-grade mesothelial proliferations and metastatic clear cell renal cell carcinomas. Analogous lesions have been described in the female genital tract, often posing diagnostic challenges due to their low incidence. Here, we present the difficult diagnostic aspects of the first case of clear cell papillary cystadenoma involving the ovary, including the salient immunohistochemical, ultrastructural, and molecular characteristics.

Reporte del primer caso de cistoadenoma papilar de epidídimo en Colombia, Hospital Universitario de Santander / Report of the first case of papillary cystadenoma of the epididymis in Colombia

Resumen La presencia de una masa en la región escrotal comprende un amplio espectro de patologías, unas con mayor urgencia para el manejo que otras, dado el riesgo asociado de pérdida testicular o desarrollo de cáncer. Dentro de las posibilidades diagnósticas se encuentran las neoplasias, destacando las originadas en el epidídimo, con un 75% de casos de etiología benigna, correspondiendo un 9% al cistoadenoma papilar de epidídimo que sobresale por su baja frecuencia dado que en la literatura mundial existen aproximadamente 70 reportes publicados con escasos provenientes de literatura médica en castellano, en los que se incluye el presente caso expuesto en el que se confirma histopatológicamente un cistoadenoma papilar de epidídimo y se presenta una revisión del tema acerca del enfoque de un paciente con masa escrotal. Asimismo se resalta el hecho de que esta lesión puede asociarse con la enfermedad de Von Hippel Lindau, sobre todo en presentaciones bilaterales. MÉD.UIS. 2020;33(2):95-100.

Abstract The presence of a mass in the scrotal region comprises a wide spectrum of pathologies, some with greater urgency for management than others, given the associated risk of testicular loss or cancer development. Among the diagnostic possibilities are the neoplasms, highlighting those originating in the epididymis, with 75% of cases of benign etiology, 9% corresponding to the papillary cystadenoma of the epididymis that stands out for its low frequency given that in the world literature there are approximately 70 published reports with few from medical literature in Spanish, which include the present case presented in which a papillary cystadenoma of the epididymis is confirmed histopathologically and a review of the topic about the approach of a patient with scrotal mass is presented. Also the fact that this lesion can be associated with von Hippel Lindau disease, especially in bilateral presentations is highlighted. MÉD.UIS. 2020;33(2):95-100.

Synchronous Oncocytic Papillary Cystadenoma and Warthin Tumor of the Parotid Gland.

Warthin tumor (WT) is the second most common benign salivary gland neoplasm. It is also the most frequent salivary gland tumor to occur synchronously or metachronously with another salivary gland neoplasm, in the same gland or on the other side. Oncocytic papillary cystadenomas (OPCs) are rare neoplasms that are more common in minor salivary gland locations and in women. We describe in detail the case of a 73-year-old male smoker with synchronous OPC and WT of the parotid gland. On microscopy, both tumors resembled each other considerably, with the only difference being that the OPC lacked the tumor-associated lymphoid proliferation characteristic of WT. These findings highlight that OPC bears considerable similarity to WT. While this morphological similarity may lead to misdiagnosis on rare occasions, it does not affect patient management, as clinical behavior of both these tumors is similar.

Nevo de Jadassohn asociado a siringocistoadenoma papilífero / Associated Jadessohn's nevus syringocistoadenoma papilífero

El siringocistoadenoma papilífero (SCAP) es un tumor anexial benigno, poco frecuente, que deriva de las glándulas sudoríparas écrinas o apócrinas. Afecta a ambos sexos por igual, se observa desde el nacimiento o en la primera infancia. Se manifiesta como una placa solitaria alopécica en cuero cabelludo, o como pápulas de color piel en rostro y cuello. En el 40% de los casos se asocia a un nevo sebáceo preexistente. El tratamiento de elección es la extirpación quirúrgica. Se presenta un varón de 14 años, con un nevo de Jadassohn presente desde el nacimiento, que desarrolló años posteriores un SCAP. Palabras clave Nevo de Jadassohn, nevo sebáceo, siringocistoadenoma papilífero, tumor anexial benigno.

The syringocystadenoma papilliferum (SCAP) is an adnexal benign tumor, uncommon, which derives from the eccrine or apocrine sebaceous glands. It affects both sexes equally, it occurs most frequently from birth or early childhood. It manifests clinically as a solitary raised plate located mainly on scalp, or as papules in face, and neck. In 40% of the cases it is associated with a pre-existing sebaceous nevus. The treatment of choice is surgery. We present a 14 year old male, with a history of a nevus of Jadassohn present from birth, that years later developed a SCAP

Bilateral submandibular gland nodular oncocytic hyperplasia with papillary cystadenoma-like areas.

A 57-year-old man presented in 2016 with a 4-month history of a right submandibular mass, having undergone left submandibular gland (SMG) excision in 2003. Imaging suggested a benign tumour and subsequent core biopsy findings suggested a nodular oncocytic hyperplasia, similar to the tumour removed from the contralateral side. This was confirmed on histological analysis following right submandibular gland excision which showed characteristic features of nodular oncocytic hyperplasia along with an unusual diffuse papillary cystadenoma-like ductal proliferation, similar to that seen in the 2003 specimen. A diagnosis of multinodular adenomatous oncocytic hyperplasia (MAOH) was rendered in order to communicate the unique histological features that have otherwise not been described in the literature. We believe that this is the first reported case of non-synchronous multinodular oncocytic hyperplasia and the first case affecting the submandibular glands.

Cistoadenoma papilar oncocítico de glándula parótida: caso clínico / Papillary oncocytic cystoadenoma of the parotid gland: case report

RESUMEN El cistoadenoma es un tumor epitelial benigno raro de glándulas salivales, de crecimiento lento y asintomático, que forma grandes masas uni o multiloculares con desarrollo papilar intraluminal. Si bien es más frecuente en glándulas salivales menores, se ha destacado lo inusual de la lesión en glándulas salivales mayores. Se presenta el caso de una mujer de 60 años, quien consultó por una masa indolora, blanda y de consistencia quistica en glándula parótida derecha, de crecimiento lento y progresivo. Su evolución fue de varios meses con piel de la zona intacta. Se realizó parotidectomia superficial. El examen histopatológico demostró cistoadenoma papilar oncocitico, tumor que ha sido destacado por su infrecuencia, y con una manifestación en parótida sumamente rara y de variados patrones histológicos. Su reconocimiento es trascendente para el profesional en casos como el que se presenta, pues su diagnóstico diferencial incluye lesiones con aspecto clínico similar y en algunos casos comportamiento agresivo.

ABSTRACT The cystadenoma is a rare benign epithelial tumor of salivary glands, characterized by slow growing and asymptomatic presentation, forming large uni or multilocular masses with intraluminal papillary development. Although it is more frequent in minor salivary glands, it has been emphasized the unusual of the major salivary glands affection. We present the case of a 60-year-old woman consulting for a painless, soft mass with cystic consistency in the right parotid gland, slow and progressive growing. The tumor evolved for several months keeping the skin of the area intact. A superficial parotidectomy was performed. Histopathological examination demonstrated a papillary oncocytic cystadenoma, a tumor that has been noted for its low frequency, and with extremely rare parotid manifestation and varied histological patterns. Its recognition is relevant for the professional in cases such as the one presented, since its differential diagnosis includes lesions with similar clinical appearance and in some cases aggressive behavior.

Unilateral Papillary Cystadenoma of the Epididymis as a First Presentation of Von Hippel-Lindau Disease.

Association between papillary cystadenoma of the epididymis (PCE) and Von Hippel-Lindau Disease (VHLD) is well known and stronger for bilateral tumors. Unilateral PCE occurs either as a sporadic tumor without evidence of VHLD or in the context of a known diagnosis of VHLD, indeed it has never been reported as the first manifestation of VHLD. In contrast, we report the case of a boy with an apparently isolated, unilateral PCE that resulted to be the first manifestation of an unknown VHLD. Thus, we recommend screening for VHLD in patients with a new diagnosis of unilateral PCE, especially if the patients are young.

Autoantibodies against HSF1 and CCDC155 as Biomarkers of Early-Stage, High-Grade Serous Ovarian Cancer.

Background: Tumor-directed circulating autoantibodies (AAb) are a well-established feature of many solid tumor types, and are often observed prior to clinical disease manifestation. As such, they may provide a good indicator of early disease development. We have conducted a pilot study to identify novel AAbs as markers of early-stage HGSOCs.Methods: A rare cohort of patients with early (FIGO stage Ia-c) HGSOCs for IgG, IgA, and IgM-mediated AAb reactivity using high-content protein arrays (containing 9,184 individual proteins). AAb reactivity against selected antigens was validated by ELISA in a second, independent cohort of individual patients.Results: A total of 184 antigens were differentially detected in early-stage HGSOC patients compared with all other patient groups assessed. Among the six most highly detected "early-stage" antigens, anti-IgA AAbs against HSF1 and anti-IgG AAbs CCDC155 (KASH5; nesprin 5) were significantly elevated in patients with early-stage malignancy. Receiver operating characteristic (ROC) analysis suggested that AAbs against HSF1 provided better detection of early-stage malignancy than CA125 alone. Combined measurement of anti-HSF1, anti-CCDC155, and CA125 also improved efficacy at higher sensitivity.Conclusions: The combined measurement of anti-HSF1, anti-CCDC155, and CA125 may be useful for early-stage HGSOC detection.Impact: This is the first study to specifically identify AAbs associated with early-stage HGSOC. The presence and high frequency of specific AAbs in early-stage cancer patients warrants a larger scale examination to define their value for early disease detection at primary diagnosis and/or recurrence. Cancer Epidemiol Biomarkers Prev; 27(2); 183-92. ©2017 AACR.

Adolescent Hydrocele Carrying a Surprise: A Case of Papillary Cystadenoma of the Epididymis.

Papillary cystadenoma of the epididymis (PCE) is a rare benign epithelial tumor remarkable for its association with von Hippel-Lindau disease. A 12-year-old boy consulted for a progressive enlargement of the left testicle. At time of surgery, the whole epididymis was enlarged. Pathologic diagnosis was PCE with a focus on borderline malignancy. Scrotal left epididymectomy was performed. von Hippel-Lindau disease screening was negative. No relapse has been detected 2 years later. In case of atypical clinical examination of a hydrocele, unusual presentations such as PCE should be considered. The main differential diagnoses were adenomatoid tumor, nonpapillary cystadenoma, and metastatic clear cell renal carcinoma.

[Papillary cystadenoma: a rare differential diagnosis of a paratesticular tumour]. / Papilläres Zystadenom als seltene Differenzialdiagnose einer paratestikulären Raumforderung.

Testicular and paratesticular cystadenomas arise from an oviduct-like structure, which, morphologically, is almost identical with the ovarian surface epithelium. These are very rare benign tumours of adults. They present as asymptomatic cystic lesions. Bilateral paratesticular cystadenomas are associated with the Von-Hippel-Lindau syndrome and may be associated with infertility. Most cystadenomas are benign, but a few cases of malignant transformation of embryonic remnants have been reported in the appendix testis, including cases of adenocarcinoma, cystadenocarcinoma, and a Müllerian-type epithelial tumour with a low malignant potential. We report the case of a 74-year-old man with a rare paratesticular cystadenoma of the male adnexa.